Coexistence of Marfan-like Connective Tissue Disease with Morphologic Left Ventricular Non-compaction.

We treated a man with co-incident Marfan-like connective tissue disease with morphologic left ventricular non-compaction (LVNC). He underwent valve-sparing aortic root replacement because of aortic root dilation at 43 years old. Pathological findings of the aorta revealed cystic medio-necrosis, consistent with Marfan syndrome. He developed congestive heart failure caused by LVNC at 47 years old. His daughter had scoliosis, and he had several physical characteristics suggestive of Marfan syndrome. We herein report a rare case of a patient who had Marfan-like connective disease with an LVNC appearance.

Citation

Satomi Yashima, Hiroyuki Takaoka, Togo Iwahana, Manami Takahashi, Yusuke Kondo, Hideki Ueda, Aya Saito, Yuya Ito, Noboru Motomura, Nobuyuki Hiruta, Jun-Ichiro Ikeda, Goro Matsumiya, Yoshio Kobayashi. Coexistence of Marfan-like Connective Tissue Disease with Morphologic Left Ventricular Non-compaction. Internal medicine (Tokyo, Japan). 2020 Dec 01;59(23):3039-3044

Mesh Tags

PMID: 32759589

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